Objectives: Extra-cardiac conduit total cavopulmonary connection (EC-TCPC) has theoretical advantages over the other types of Fontan modification. In this study the clinical results of this procedure are assessed. Methods: Since 1994, 364 patients underwent EC-TCPC in Fukuoka Children’s Hospital. Median age and body weight at operation were 3.5 years and 12.6 kg. Most of the patients are receiving coumadin and aspirin postoperatively. Surgical results and hemodynamic variables were reviewed. Results: Follow-up time was 6.0±3.9 years. There were no operative death and 13 late deaths (3.6%). Actuarial survival rate was 95.5% at 10 years and 92.2% at 14 years. Eight patients had reoperation including fenestration closure in 1, aortic valve replacement in 1, ligation of major collateral vessel in 2 and pacemaker implantation in 4. Six patients had catheter intervention for branch pulmonary stenosis. Nineteen patients developed late complication including protein loosing enteropathy in 4, thromboembolism in 3, bleeding complication in 3, and new onset supraventricular arrhythmia in 9. Freedom from Fontan-related events was 85.6% at 10 years and 83.2% at 14 years. The incidence of late complication was not related to the size of the conduit. Late cardiac catheterization for 325 patients showed central venous pressure of 9.5±2.6mmHg, cardiac index of 3.5±0.8L/kg/min and arterial oxygen saturation of 94.1±2.3%. No patient showed conduit stenosis. Plasma concentrations of atrial and brain natriuretic polypeptide (pg/ml) were 34.3±32.6 and 26.0±48.3. Conclusion: Early to midterm outcome of EC-TCPC was satisfactory with low incidence of late mortality and morbidity, and excellent hemodynamic state.